Inborn errors of metabolism:
Indications for MEPSEVII:
Treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).
Limitations of Use:
Effect on the CNS manifestations of MPS VII has not been determined.
Adults and Children:
Premedicate with a non-sedating antihistamine with or without an antipyretic 30–60mins prior to infusion. Give as IV infusion over 4hrs (infuse first 2.5% of total volume over 1hr, then increase rate as tolerated over next 3hrs); see full labeling. 4mg/kg every 2 weeks.
Have appropriate medical support readily available. Should be administered under supervision of healthcare professional. Monitor during and for ≥60mins post-infusion for anaphylaxis; discontinue immediately if a severe systemic reaction occurs. Elderly. Pregnancy. Nursing mothers.
Recombinant human lysosomal beta glucuronidase.
Infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, pruritus.
Single-dose vial (5mL)—1